Pappo Alberto S, Janeway Katherine A
Department of Pediatrics, Texas Children's Cancer Center, 6621 Fannin Street, CC1510.00, Houston, TX 77030, USA.
Hematol Oncol Clin North Am. 2009 Feb;23(1):15-34, vii. doi: 10.1016/j.hoc.2008.11.005.
Gastrointestinal stromal tumors (GISTs) rarely occur in pediatric patients, but increased recognition of adult GIST has led to better awareness of the existence of this entity in the pediatric population. GIST occurring in pediatric patients has a unique biology and clinical behavior and warrants discussion as an independent entity. The generally accepted definition of pediatric GIST is a tumor that is diagnosed at the age of 18 years or younger. This review highlights the clinical features, molecular biology, and clinical management of this rare pediatric entity.
胃肠道间质瘤(GISTs)在儿科患者中很少见,但对成人GIST认识的增加使人们更好地认识到该实体在儿科人群中的存在。发生在儿科患者中的GIST具有独特的生物学和临床行为,值得作为一个独立实体进行讨论。儿科GIST的普遍接受定义是在18岁及以下诊断出的肿瘤。本综述重点介绍了这种罕见儿科实体的临床特征、分子生物学和临床管理。